[Primary familial pulmonary arterial hypertension].
The authors present the case of three young female patients, of the same family, with all quickly fatal Primary Pulmonary Hypertension (PPH). Their clinical signs and paraclinical investigations were suggestive of a PPH and excluded all others causes of Pulmonary Hypertension. These observations recall the possible familial cases of this rare and very severe disease. It emphasizes as well the possible revelation of the disease during or after a pregnancy. So the members of this family, essentially first the young women who wish to have children, need a medical detection.
Scherpereel
A
A
Service de Soins intensifs cardiologiques, Centre Hospitalier de Roubaix.
Steenhouwer
F
F
Quandalle
P
P
Dennetière
S
S
Haftel
Y
Y
Declercq
N
N
Demarcq
J M
JM
D000328:Adult; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000235:genetics* / Q000401:mortality; D007231:Infant, Newborn; D010375:Pedigree; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular; D013902:Radiography, Thoracic
Hypertension artérielle pulmonaire primitive familiale.